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Hypoplastic left heart syndrome

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https://www.readbyqxmd.com/read/29777281/right-ventricular-systolic-function-parameters-in-hypoplastic-left-heart-syndrome
#1
S Javed Zaidi, Jamie Penk, Vivian W Cui, Supaluck Kanjanauthai, David A Roberson
Assessment of the systolic function of the right ventricle (RV) in patients with hypoplastic left heart syndrome (HLHS) is important. The asymmetric shape and heavy trabeculations make accurate assessment of RV systolic function challenging. Novel measures of RV function could be helpful in distinguishing reduced versus preserved function in HLHS and may also be worse in HLHS with preserved function compared to normal controls. These novel methods offer promise, but research and clinical applicability is hindered as no cut-off values for normal function have been established...
May 18, 2018: Pediatric Cardiology
https://www.readbyqxmd.com/read/29774394/longitudinal-health-care-cost-in-hypoplastic-left-heart-syndrome-palliation
#2
Jesse E Hansen, Nicolas L Madsen, Laurie Bishop, David L S Morales, Jeffrey B Anderson
Management of hypoplastic left heart syndrome (HLHS) is resource intensive. Heath care systems are pressured to provide value to patients by improving outcomes while decreasing costs. A single-center retrospective cohort of infants with HLHS who underwent Norwood procedure or hybrid Norwood from 2004 to 2014 and survived to first outpatient follow up were studied. The primary outcome was total cost through 12 months with a sub-analysis of patients with 60 months of data. Costs were calculated using internal cost accounting system and reported by cost center...
May 17, 2018: Pediatric Cardiology
https://www.readbyqxmd.com/read/29770299/regenerative-medicine-therapy-for-single-ventricle-congenital-heart-disease
#3
REVIEW
Chetan Ambastha, Gregory J Bittle, David Morales, Nathaniel Parchment, Progyaparamita Saha, Rachana Mishra, Sudhish Sharma, Alexander Vasilenko, Muthukumar Gunasekaran, Manal T Al-Suqi, Deqiang Li, Peixin Yang, Sunjay Kaushal
One of the most complex forms of congenital heart disease (CHD) involving single ventricle physiology is hypoplastic left heart syndrome (HLHS), characterized by underdevelopment of the left ventricle (LV), mitral and aortic valves, and narrowing of the ascending aorta. The underdeveloped LV is incapable of providing long-term systemic flow, and if left untreated, the condition is fatal. Current treatment for this condition consists of three consecutive staged palliative operations: the first is conducted within the first few weeks of birth, the second between 4 to 6 months, and the third and final surgery within the first 4 years...
April 2018: Translational Pediatrics
https://www.readbyqxmd.com/read/29756326/gastrostomy-tube-placement-among-infants-with-hypoplastic-left-heart-syndrome-undergoing-stage-1-palliation
#4
Parthak Prodhan, Xinyu Tang, Jeffrey Gossett, Brandon Beam, Janet Simsic, Nancy Ghanayem, Nahed O ElHassan
OBJECTIVE: Different feeding strategies have been suggested to improve growth and survival of infants with hypoplastic left heart syndrome following stage 1 palliation. The study objective was to assess hospital mortality following stage 1 palliation among infants with hypoplastic left heart syndrome who had two feeding modalities, gastrostomy tube vs no gastrostomy tube. DESIGN: Retrospective study design. SETTING: Multicenter pediatric heath information system database...
May 13, 2018: Congenital Heart Disease
https://www.readbyqxmd.com/read/29754919/balloon-expandable-stents-for-recoarctation-of-the-aorta-in-small-children-two-centre-experience
#5
K Gendera, P Ewert, D Tanase, S Georgiev, T Genz, P Bambul Heck, T Moszura, I Malcic, J Cleuziou, A Eicken
BACKGROUND: In young patients with native aortic coarctation (CoA), the management of choice is surgery. However, in re-coarctation (re-CoA) surgery is associated with increased morbidity and even mortality. Some children with native CoA present relative contraindications for surgery. METHODS: From 2006 to 2017, thirty-four patients (male n = 20; 59%) from two centres with re-CoA (31) and native CoA (3) were managed by stent implantation with premounted balloon expandable stents...
July 15, 2018: International Journal of Cardiology
https://www.readbyqxmd.com/read/29754202/regional-differences-in-cost-and-length-of-stay-in-neonates-with-hypoplastic-left-heart-syndrome
#6
Luma Essaid, Paula D Strassle, Eric G Jernigan, Jennifer S Nelson
Hypoplastic left heart syndrome (HLHS) is a highly resource-intensive diagnosis. Geographic variation in cost and length of stay (LOS) in HLHS is not well described. Neonates diagnosed with HLHS between 2000 and 2012 were identified using the Kids' Inpatient Database. Hospitalizations were stratified into two groups: (1) birth and (2) secondary. United States regional differences in hospital charges and LOS were compared using adjusted linear regression. Of 2431 birth hospitalizations, 449 neonates (18.5%) died while inpatient and mortality rates differed by region (p = 0...
May 12, 2018: Pediatric Cardiology
https://www.readbyqxmd.com/read/29748699/longitudinal-deformation-of-the-right-ventricle-in-hypoplastic-left-heart-syndrome-a-comparative-study-of-2d-feature-tracking-magnetic-resonance-imaging-and-2d-speckle-tracking-echocardiography
#7
Mona Salehi Ravesh, Carsten Rickers, Finn Jonathan Bannert, David Hautemann, Abdullah Al Bulushi, Dominik Daniel Gabbert, Philip Wegner, Eva Kis, Jan Hinnerk Hansen, Michaeal Jerosch-Herold, H-H Kramer, Jana Logoteta
In hypoplastic left heart syndrome (HLHS), long-term outcome is closely related to right ventricular function. Echocardiography and magnetic resonance imaging (MRI) are routinely used for functional assessment. MRI 2D-tissue feature tracking (2D-FT) allows quantification of myocardial deformation but has not yet been applied to HLHS patients. We sought to investigate the feasibility of this technique and to compare the results to 2D-speckle tracking echocardiography (2D-STE). In routine MRI 2D anatomical four chamber view, cine images were recorded in 55 HLHS patients (median age 4...
May 10, 2018: Pediatric Cardiology
https://www.readbyqxmd.com/read/29712484/does-prenatal-diagnosis-of-critical-congenital-heart-diseases-influence-the-prereferral-mortality-in-a-center-without-surgical-intervention
#8
Buse Özer Bekmez, Evrim Alyamaç Dizdar, Nilüfer Okur, Mehmet Büyüktiryaki, Nurdan Uraş, Serife Suna Oğuz
BACKGROUND: Prenatal diagnosis ameliorates some preoperative and postoperative outcomes in critical congenital heart disease (CHD). Despite large variability among anatomical defect types, nearly half of them are diagnosed antenatally. We aimed to investigate the effect of the antenatal diagnosis on prereferral mortality of infants with critical CHD in a center without cardiovascular surgery clinic. METHODS: Medical records of the neonates who were diagnosed with critical CHD between the years 2010 and 2016 in Zekai Tahir Burak Women's health Education and Research Hospital were retrospectively reviewed for the study...
April 30, 2018: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/29702180/hypoplastic-left-heart-syndrome-with-a-hypoplastic-lung-in-congenital-diaphragmatic-hernia-what-should-be-done-first
#9
EDITORIAL
Shu-Chien Huang, Wen-Ming Hsu
No abstract text is available yet for this article.
April 24, 2018: Seminars in Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29683008/first-trimester-combined-screening-biochemistry-in-detection-of-congenital-heart-defects
#10
Julia Alanen, Teemu Korpimaki, Heikki Kouru, Mikko Sairanen, Markku Leskinen, Mika Gissler, Markku Ryynanen, Jaana Nevalainen
OBJECTIVE: To evaluate the performance of first trimester biochemical markers, pregnancy-associated plasma protein-A (PAPP-A), free beta human chorionic gonadotropin (fβ-hCG), and nuchal translucency (NT) in detection of severe congenital heart defects (CHDs). METHODS: During the study period from 1 January 2008 to 31 December 2011, biochemical markers and NT were measured in 31,144 women as part of voluntary first trimester screening program for Down's syndrome in Northern Finland...
April 22, 2018: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/29681546/educational-series-in-congenital-heart-disease-congenital-left-sided-heart-obstruction
#11
REVIEW
Michelle Carr, Stephanie Curtis, Jan Marek
Congenital obstruction of the left ventricular outflow tract remains a significant problem and multilevel obstruction can often coexist. Obstruction can take several morphological forms and may involve the subvalvar, valvar or supravalvar portion of the aortic valve complex. Congenital valvar stenosis presenting in the neonatal period represents a spectrum of disorders ranging from the hypoplastic left heart syndrome to almost normal hearts. Treatment options vary dependent on the severity of the left ventricular outflow tract obstruction (LVOTO) and the variable degree of left ventricular hypoplasia as well as the associated lesions such as arch hypoplasia and coarctation...
June 2018: Echo Research and Practice
https://www.readbyqxmd.com/read/29679990/three-dimensional-echocardiographic-evaluation-of-ebstein-s-anomaly-of-the-tricuspid-valve-in-a-patient-with-hypoplastic-left-heart-syndrome
#12
Courtney Cassidy, Max B Mitchell, Pei-Ni Jone
We report three-dimensional imaging of a rare finding of Ebstein's anomaly of the tricuspid valve in a patient with hypoplastic left heart syndrome, which has been previously reported only by two-dimensional echocardiography. A fetal echocardiogram was performed at 19 weeks that showed a moderately hypoplastic left ventricle, severely hypoplastic mitral valve, a severely hypoplastic aortic valve, and a dysplastic tricuspid valve. Post Caesarean delivery at 40 weeks of gestation, a transthoracic echocardiogram confirmed the findings seen on the fetal echocardiogram...
April 22, 2018: Cardiology in the Young
https://www.readbyqxmd.com/read/29664500/nutritional-recovery-after-cardiac-surgery-in-children-with-congenital-heart-disease
#13
I Oyarzún, C Claveria, G Larios, C Le Roy
INTRODUCTION: Malnutrition is common in children with congenital heart disease (CHD). Medical treatment and surgical interventions contribute improving the nutritional status of these children. OBJECTIVE: To describe nutritional recovery in children with CHD and associated factors after surgery. PATIENTS AND METHOD: Longitudinal study. 46 Children under 18 years old admitted for CHD surgery between April 2015 and April 2016 were recruited...
February 2018: Revista Chilena de Pediatría
https://www.readbyqxmd.com/read/29655509/reduced-right-ventricular-fractional-area-change-strain-and-strain-rate-before-bidirectional-cavopulmonary-anastomosis-is-associated-with-medium-term-mortality-for-children-with-hypoplastic-left-heart-syndrome
#14
Lily Q Lin, Jennifer Conway, Silvia Alvarez, Benjamin Goot, Jesus Serrano-Lomelin, Timothy Colen, Edythe B Tham, Shelby Kutty, Ling Li, Nee Scze Khoo
BACKGROUND: Ventricular dysfunction is associated with increased morbidity and mortality in children with hypoplastic left heart syndrome. The aim of this study was to assess the diagnostic performance of conventional and speckle-tracking echocardiographic measures of right ventricular (RV) function before bidirectional cavopulmonary anastomosis palliation in predicting death or need for heart transplantation (HTx). METHODS: RV fractional area change (RVFAC) and longitudinal and circumferential strain and strain rate (SR) were measured in 64 prospectively recruited patients with hypoplastic left heart syndrome from echocardiograms obtained before bidirectional cavopulmonary anastomosis surgery...
April 12, 2018: Journal of the American Society of Echocardiography
https://www.readbyqxmd.com/read/29649824/end-organ-saturation-differences-in-early-neonatal-transition-for-left-versus-right-sided-congenital-heart-disease
#15
Gabriel Altit, Shazia Bhombal, Theresa A Tacy, Valerie Y Chock
BACKGROUND: For neonates with congenital heart disease (CHD), left-sided (LL) and right-sided (RL) single ventricular physiologies (LL, hypoplastic left heart syndrome; RL, tricuspid atresia or pulmonary atresia with intact ventricular septum) may demonstrate distinct changes in tissue saturation in the first 72 h of life. Near-infrared spectroscopy (NIRS) can measure regional cerebral saturation (Csat) and renal saturation (Rsat) to clarify differences between LL and RL over time. OBJECTIVES: Our primary objective was to measure changes in Csat and Rsat in the first 72 h of life using NIRS between CHD infants with LL compared to RL...
April 12, 2018: Neonatology
https://www.readbyqxmd.com/read/29609227/-characteristics-and-prognosis-of-interrupted-inferior-vena-cava-with-azygous-continuation
#16
Q Xu, H X Sun, J S Xie, J L Wang, Q Y Pei, X H Zhang
Objective: To explore the clinical characteristics of interrupted of the inferior vena cava with azygous continuation and the prognosis. Methods: Retrospective analysis of 21 fetuses diagnosed with interrupted inferior vena cava with azygous continuation among 28 567 pregnant women who underwent routine ultrasound scan. The clinical data, ultrasonographic features, genetic information and prognosis were collected. Results: Interrupted of the inferior vena cava with azygous continuation occurred in 21(0.07%, 21/28 567) of 28 567 patients...
March 25, 2018: Zhonghua Fu Chan Ke za Zhi
https://www.readbyqxmd.com/read/29580790/recognition-of-neonatal-lymphatic-flow-disorder-fetal-mr-findings-and-postnatal-mr-lymphangiogram-correlation
#17
David M Biko, Jordan A Johnstone, Yoav Dori, Teresa Victoria, Edward R Oliver, Maxim Itkin
RATIONALE AND OBJECTIVES: This study aimed to describe prenatal and postnatal imaging features and outcomes of neonates with neonatal lymphatic disorders (NLDs). MATERIALS AND METHODS: An institutional review board-approved search of the radiology database for patients with NLD identified five patients. Inclusion criteria include prenatal imaging (fetal magnetic resonance [MR] imaging and ultrasound) and postnatal three-dimensional T2 Sampling Perfection with Application optimized Contrasts using different flip angle Evolution (SPACE) and dynamic contrast-enhanced MR lymphangiography within 6 months of life...
March 23, 2018: Academic Radiology
https://www.readbyqxmd.com/read/29577004/staged-surgical-palliation-for-hlhs-in-a-girl-with-severe-factor-x-deficiency
#18
Ahmed F Elmahrouk, Mohamed F Ismail, Abdulbadee Bugis, Nashwa Badawy, Hesham Mohamed Aboelghar, Tamer Hamouda, Ahmed Jamjoom
Background  Factor X deficiency (also known as Stuart-Prower factor deficiency) is an autosomal recessive extremely rare hereditary hematologic disorder, affecting around 1:1,000,000 of the general population. Case Presentation  This case report describes a patient with hypoplastic left heart syndrome and severe factor X deficiency, who underwent staged surgical palliation. From stage 1 Norwood palliation, through superior cavopulmonary anastomosis and ending with total cavopulmonary connection with satisfactory hemostasis and no significant perioperative bleeding complication...
January 2018: Thoracic and Cardiovascular Surgeon Reports
https://www.readbyqxmd.com/read/29571602/heart-failure-after-the-norwood-procedure-an-analysis-of-the-single-ventricle-reconstruction-trial
#19
William T Mahle, Chenwei Hu, Felicia Trachtenberg, JonDavid Menteer, Steven J Kindel, Anne I Dipchand, Marc E Richmond, Kevin P Daly, Heather T Henderson, Kimberly Y Lin, Michael McCulloch, Ashwin K Lal, Kurt R Schumacher, Jeffrey P Jacobs, Andrew M Atz, Chet R Villa, Kristin M Burns, Jane W Newburger
BACKGROUND: Heart failure results in significant morbidity and mortality in young children with hypoplastic left heart syndrome (HLHS) after the Norwood procedure. METHODS: We studied subjects enrolled in the prospective Single Ventricle Reconstruction (SVR) Trial who survived to hospital discharge after a Norwood operation and were followed up to age 6 years. The primary outcome was heart failure, defined as heart transplant listing after Norwood hospitalization, death attributable to heart failure, or symptomatic heart failure (New York Heart Association [NYHA] Class IV)...
February 16, 2018: Journal of Heart and Lung Transplantation
https://www.readbyqxmd.com/read/29569026/the-genetic-landscape-of-hypoplastic-left-heart-syndrome
#20
Hisato Yagi, Xiaoqin Liu, George C Gabriel, Yijen Wu, Kevin Peterson, Stephen A Murray, Bruce J Aronow, Lisa J Martin, D Woodrow Benson, Cecilia W Lo
Hypoplastic left heart syndrome (HLHS) is one of the most lethal congenital heart defects, and remains clinically challenging. While surgical palliation allows most HLHS patients to survive their critical heart disease with a single-ventricle physiology, many will suffer heart failure, requiring heart transplantation as the only therapeutic course. Current paradigm suggests HLHS is largely of hemodynamic origin, but recent findings from analysis of the first mouse model of HLHS showed intrinsic cardiomyocyte proliferation and differentiation defects underlying the left ventricular (LV) hypoplasia...
March 22, 2018: Pediatric Cardiology
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